INTRODUCTION	Section 2 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

Background: Temporal arteritis originates from a generalized vasculopathy affecting medium and large arteries. New-onset headache in any patient older than 50 years prompts consideration of this diagnosis.

Early recognition and treatment remain critical to prevent monocular or binocular blindness, as first reported in the literature in 1938.

Pathophysiology: Confusion surrounds the terminology used to classify this vasculitis. Temporal arteritis is a commonly used term, as inflammation often strikes the temporal artery, causing temporal-located headaches. In addition, findings from biopsy of the superficial temporal artery assist in establishing the diagnosis.

Giant cell arteritis, an alternative name for this disorder, describes the pathologic appearance of affected vessels infiltrated by lymphocytes, plasma cells, and multinucleated giant cells. Patchy or segmental changes overcome medium and large arteries of the head and neck and may extend into the carotids and aorta.

A cell-mediated immune response (oligoclonal T-cell expansion in peripheral blood lymphocytes) directed toward antigens in or near elastic tissue component of the arterial wall may account for this disorder. Pathologic alterations encountered with temporal arteritis also occur in other conditions, most notably polymyalgia rheumatica (PMR). However, PMR coexists with other disorders, and random biopsies of temporal arteries reveal positive findings in only 20% of patients with PMR.

Cytokine patterns of vasculitic lesions may help distinguish temporal arteritis, PMR, and additional inflammatory conditions.

Visual impairment results from inflammation of branches of the ophthalmic artery, particularly the posterior ciliary artery, leading to ischemic optic neuritis. Additionally, the central retinal artery is affected, and almost one half of cases involve loss of eyesight.

Frequency:

• In the US: Temporal arteritis occurs relatively frequently, particularly in patients older than 50 years, with the occurrence ranging from 10 in 100,000 to 50 in 100,000.

• Internationally: Incidence rates appear higher in northern climates.

Mortality/Morbidity:

• Long-term survival of patients with temporal arteritis is the same as for the general population.

• Visual loss is the most worrisome morbidity associated with temporal arteritis, affecting as many as 50% of patients who are untreated.

• As many as 75% of patients experiencing visual disturbance in 1 eye develop visual loss in the contralateral eye within 3 weeks.

Race: Temporal arteritis occurs more frequently in Caucasian patients of northern European descent, yet rarely in blacks and Asians.

Sex: Women develop temporal arteritis 4-6 times more frequently than men.

Age: Temporal arteritis rarely occurs in patients younger than 50 years, with the mean age of onset at 70 years. High incidence, approaching 1%, exists in all patients older than 80 years.

	CLINICAL	Section 3 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

History:

• Headache, the most universal symptom, presents in more than 85% of patients with temporal arteritis. It tends to be new or change in character from a previous headache pattern, yet few features distinguish a headache caused by temporal arteritis from those arising from other etiologies.

• Cephalgia tends to be unilateral, classically concentrating in the temporal region, but may present in a diffuse or bilateral distribution.

• Onset is apt to be indolent. It is frequently worse at night, and the headache gradually worsens over several days.

• Underlying pain described as a burning or jabbing sensation may involve the surface of the scalp and face more than intracranial components.

• Simple acts such as combing hair or resting the head on a pillow exacerbate pain, with severity precluding the wearing of a hat or glasses.

• Vision loss may present as the first symptom, with approximately 50% of patients complaining of impaired vision.

• Onset may occur suddenly and painlessly.

• Loss may be transient (amaurosis fugax-type picture) or permanent. Initial ocular disturbances may fluctuate, but blindness eventually occurs in 20-50% of untreated patients.

• Bilateral visual loss befalls more than 50% of untreated patients; the second eye usually is affected within 2-3 weeks of the first.

• Jaw claudication is especially prominent when patient is chewing or talking and occurs in more than one half (approximately 65%) of patients with temporal arteritis.

• Constitutional symptoms include anorexia and weight loss, fever and sweats, and malaise, fatigue, and depression.

• Based on the 1990 American College of Rheumatology criteria for classification of giant cell arteritis, 3 of the following 5 items must be present:

• Development of symptoms in patients older than 50 years

• New onset of headache or localized head pain

• Temporal artery tenderness to palpation

• Decreased pulsations not related to arteriosclerosis of cervical arteries

• Westergren erythrocyte sedimentation rate (WESR) greater than 50 mm/h

Physical: Perform a general physical examination to exclude other possible serious causes of headache and visual disturbance.

• A low-grade fever and mild meningeal signs may accompany temporal arteritis.

• Carefully examine the head and face.

• Palpation of affected artery may reveal exquisite tenderness, warmth, and pulselessness.

• Inflamed arteries may be dilated and thickened, allowing vessels to be rolled between the fingers and skull.

• Inspect for tender scalp nodules or necrotic spots that may appear over any part of the scalp.

• Check for cranial nerve palsies (especially sixth nerve palsy).

• General eye examination is as follows:

• Check visual acuity and visual fields; vision loss may be profound, and the patient may be limited to counting fingers or worse.

• Test for an afferent pupillary defect.

• Slit lamp examination helps exclude other potential problems.

• Funduscopic inspection is as follows:

• This may appear normal.

• Optic disc may appear pale and edematous with temporal arteritis.

• Screen for scattered cotton-wool patches and small flame hemorrhages.

• Look for distended, beaded retinal veins with temporal arteritis.